Hypothalamic juvenile pilocytic astrocytoma presenting with intracerebral hemorrhage

Hemorrhage of juvenile pilocytic astrocytomas is very rare. However, it is clinically important because a high probability of a fatal outcome exists if hemorrhage occurs outside the optic nerve. Hemorrhage of a hypothalamic juvenile pilocytic astrocytoma has been reported only once, from an autopsy examination. We present a successfully managed case. The patient, a 34-year-old man, presented with sudden loss of consciousness. Computed tomography (CT) and magnetic resonance imaging demonstrated an exophytic hypothalamic tumor with a hemorrhage. Craniotomy with total removal of the tumor and the hematoma was performed. Histopathologic examination revealed that the tumor was a juvenile pilocytic astrocytoma. Brain CT examination 18 months later revealed no evidence of tumor recurrence. The patient was well, without neurologic deficit, and had a normal social life as of the last follow-up examination 22 months after craniotomy.     

Pilocytic astrocytoma with subarachnoid dissemination

Pilocytic Astrocytomas (WHO I) are histopathologically tumors of glial origin occurring predominantly in childhood and adolescence. Normally, they are characterized by a benign clinical course, with a long overall survival time and a high rate of complete remission. The rare case of pilocytic astrocytoma, primarily located in the third ventricular region, with generalized subarachnoidal spread is described. In the 10 years of follow-up, the histopathologic findings of the seedings remained those of a typical pilocytic astrocytoma; tumor progression did not occur.     

Hypothalamic pilocytic astrocytoma presenting with intratumoral and subarachnoid hemorrhage

A 45-year-old male presented with sudden onset of severe headache. Computed tomography and magnetic resonance imaging demonstrated an irregularly enhanced suprasellar mass with intratumoral and subarachnoid hemorrhage. The mass was removed in two operations. Histological examination of the tumor revealed pilocytic astrocytoma. The relatively rich vascularity and perivascular tumor cell proliferation observed in this benign lesion were probably the causes of this extremely rare association.     

Childhood fibrous dysplasia presenting as blindness: a skull base approach for resection and immediate reconstruction

Fibrous dysplasia is an abnormal fibroosseous process of bone of unknown cause. The incidence of skull involvement varies, painless enlargement being the most common presenting symptom. Change in vision is a rare but recognized finding. We report a 3-year-old boy with extreme fibrous dysplasia involving the skull base, who presented with blindness. He underwent exposure osteotomies of the frontal bones and orbits to provide access for skull base tumor removal. The orbital roofs were reconstructed with microplate-fixed cranial grafts. One and one half years after tumor excision followed by immediate reconstruction, the boy retains facial symmetry, and his ocular function has not deteriorated.     

Treatment of peptic ulcer

AIMS: To establish whether MIB-1 and p53 staining are useful for differentiating pilocytic astrocytomas and astrocytomas from anaplastic astrocytomas and glioblastomas. This study was restricted to children and young adults under 30 years of age because of the differences in p53 mutations between paediatric and adult astrocytomas. METHODS AND RESULTS: Forty-five astrocytic tumours, including 18 pilocytic astrocytomas, 14 astrocytomas, four anaplastic astrocytomas and nine glioblastomas, from 45 children and young adults, between 1 and 29 years (mean 11 years) of age, were examined pathologically, and sections from paraffin-embedded blocks were used for MIB-1 and p53 immunostaining. The MIB-1 labelling index and the frequency and intensity of p53 staining in both the pilocytic astrocytoma and the astrocytoma group were significantly lower than in the anaplastic astrocytoma plus glioblastoma group (P < 0.001). In 11.1% (two of 18) of pilocytic astrocytomas and 42.9% (six of 14) of astrocytomas, immunoreactivity of either MIB-1 or p53 staining was of almost the same intensity as that of anaplastic astrocytomas and glioblastomas. However, in these cases, results using both MIB-1 and p53 stain differed from those for anaplastic astrocytomas and glioblastomas. CONCLUSIONS: MIB-1 and p53 co-staining is very useful for differentiating pilocytic astrocytomas and astrocytomas from anaplastic astrocytomas and glioblastomas. However, MIB-1 or p53 staining alone cannot differentiate pilocytic astrocytomas and astrocytomas from anaplastic astrocytomas and glioblastomas.     

Recurrence of astrocytoma of optic nerve after 48 years

A tumour presumed to be a juvenile glioma of the optic nerve was removed by a Kronlein procedure from a 4-year old boy in 1925. In 1973 a severe proptosis made it necessary to remove the atrophic eye and a large retro-ocular tumour. Histological examination, supported by electron microscopy, showed the second tumour to be an anaplastic pilocytic astrocytoma. The possible histogenesis and this unusual behaviour pattern are discussed.     

Medication control in hospitals: a practical approach to the problem of drug-drug interactions

We present 19 patients with tuberous sclerosis complex and subependymal giant cell astrocytoma. The mean age at the time of tumor diagnosis was 9.4 years (range, 1.5 to 21 years). Computed cranial tomography (CT) or cranial magnetic resonance imaging (MRI) identified the lesion which was resected in all cases. Seven patients had hydrocephalus and there was an interval increase in the tumor size or a large tumor without hydrocephalus in 12 patients. Surgical criteria included: (1) presence of hydrocephalus; (2) interval increase in tumor size; (3) new focal neurologic deficit attributable to the tumor; and/or (4) symptoms of increased intracranial pressure. Eight patients were identified through a surveillance program involving annual computed cranial tomography. All of these eight patients had their tumor removed prior to the development of symptoms, none had neurologic deficits which persisted after surgery, and none has so far developed recurrent subependymal giant cell astrocytoma. In contrast, of the 11 patients from the non-surveillance group 7 were symptomatic at tumor diagnosis, 1 had a complicated postoperative course, 2 developed recurrent giant cell astrocytoma, and 1 had an extensive lesion that could not be completely excised. Periodic cranial imaging may help to identify subependymal giant cell astrocytomas in tuberous sclerosis patients before they become symptomatic. Earlier diagnosis and treatment could reduce surgical morbidity and the risk of tumor recurrence.     

Prognostic factors in malignant gliomas

The most important prognostic factor in malignant gliomas is histopathological diagnosis of the tumor. The survival of patients with anaplastic astrocytoma is much longer than that of patients with glioblastoma. The median survival of the former has been improved up to almost 4 years by the recent progress of multidisciplinary treatment, whereas that of the latter has still remained in less than 1.5 years. Other important factors proved to be associated with survival of patients with malignant gliomas are the age of patients, Karnofsky performance status on admission, surgery, radiotherapy and chemotherapy. There is substantial evidence suggesting an association between younger patient age and longer survival in adults with supratentorial anaplastic astrocytoma as well as glioblastoma. It is also consistent with evidence that the patients with better performance status on admission live longer after treatment. Gross total resection of supratentorial anaplastic astrocytoma is directly associated with longer and better survival when compared to subtotal or partial resection. For glioblastoma, however, gross total resection has not been proved to have a significant survival advantage over subtotal or partial removal. Radiotherapy has been proved to be associated with longer survival of patients with supratentorial anaplastic astrocytoma and glioblastoma. Chemotherapy has not proved effective in prolonging the survival of patients with glioblastoma. Multidrug chemotherapy with CCNU, procarbazine and vincristine has proved to have significant survival advantage over BCNU alone, suggesting chemotherapy is also a prognostic factor in patients with anaplastic astrocytoma.     

Pilocytic astrocytomas of the posterior fossa. A follow-up study in 33 patients

The extent of resection in pilocytic astrocytoma of the posterior fossa often remains undefined and the indications for further treatment in incompletely resected tumours are a matter of debate. It has been also realized that the problem of hydrocephalus in patients with pilocytic astrocytoma of the posterior fossa has not yet been solved and the diagnostic impact of postoperative CT findings remains questionable. We retrospectively reviewed the data from 33 patients harbouring a pilocytic astrocytoma of the posterior fossa to evaluate the impact of surgical technique in terms of radicality and of postoperative imaging results upon prognosis and adjunctive treatment. In addition, the issue of hydrocephalus was considered and related to different treatment modalities. Thirty patients underwent surgical treatment whereas 3 had open biopsy of the tumour. Macroscopically gross total resection of the tumour was performed in 20 patients, whereas resection was partial in 10. Follow-up was obtained in 29 patients for a period which ranged between 2 and 184 months (85 months +/- 56 months). Outcome was good in 24 patients who had only slight neurological deficit and poor in 3 patients, who were severely disabled. Two patients died during the follow-up period. Recurrent tumour growth occurred in 2 cases with incompletely resected tumours. From the series presented, it was concluded that long-term follow-up with CT seems mandatory in cases with contrast-enhancing residual tumour. Recurrent tumour growth should be assumed in postsurgical patients with an enlarging area of enhancement shown in follow-up CT studies. Permanent ventriculoperitoneal shunting is required in certain patients with pre- or postoperative hydrocephalus.(ABSTRACT TRUNCATED AT 250 WORDS)     

Giant cephalhematoma in a 15-year-old boy. Unilateral amaurosis as the main complication

BACKGROUND: The term "cephalhematoma" is applied to a collection of blood under the periosteum of a skull bone. It most frequently occurs in the neonatal period and is rare at other times of life. METHODS AND RESULTS: We report on a 15-year-old boy with a subperiosteal hematoma involving the whole cranial surface. Surgical drainage was performed. In the immediate postoperative course, the patient developed a progressive decrease in the right eye visual acuity. An orbital computed tomographic scan revealed the retro-orbital subperiosteal extension of the hematoma. A second surgical procedure was necessary to obtain visual recovery. CONCLUSIONS: The occurrence of cephalhematoma in adults is exceptional. However, it may lead to serious complications. Surgical treatment should be aimed to avoid retro-orbital extension of the hematoma.     

Genetics of pediatric central nervous system tumors

PURPOSE: Pediatric central nervous system (CNS) tumors comprise a wide variety of histologic subtypes ranging from the benign juvenile pilocytic astrocytoma to the highly aggressive atypical teratoid/rhabdoid tumor. Although some brain tumors are seen in association with inherited genetic disorders which predispose to malignancies, most are sporadic. Current knowledge regarding the cytogenetic and molecular genetic events which have been implicated in the development or progression of common brain tumors in children in the subject of this review. METHODS: Combined cytogenetic and molecular genetic approaches, including fluorescence in situ hybridization, have been used to identify genomic alterations in different histologic types of pediatric brain tumors. RESULTS: The most frequent abnormality in primitive neuroectodermal tumor/medulloblastoma is an i(17q), present in approximately 50% of cases. This finding implicates the presence of a tumor suppressor gene on 17p, which is important in tumor development. A number of genes on 17p have been eliminated as candidates for this locus, including TP53. A tumor suppressor gene in chromosome band 22q11.2 has been hypothesized to play a role in atypical teratoid/rhabdoid tumors, and positional cloning strategies are in progress to identify a rhabdoid tumor gene. Chromosome 22 deletions are also seen in meningiomas and a small percentage of ependymomas, but it is not yet known whether the same gene is responsible for more than one malignancy. With regard to childhood astrocytomas, tumor-associated genetic changes have not yet been identified for the common juvenile pilocytic or low grade diffuse astrocytoma. In contrast, malignant anaplastic astrocytomas and glioblastoma multiforme have abnormalities similar to those seen in adults, including loss of alleles on 17p13 and TP53 mutations, trisomy 7, EGFR rearrangements, and loss of chromosomes 10 and 22. CONCLUSIONS: The presence of tumor-associated genetic abnormalities has clinical utility in a differential diagnostic setting, and has lead to the identification of genes which contribute to tumorigenesis.     

Molecular biology of pediatric gliomas

The genes involved in the genesis and progression of adult astrocytic tumors have been an area of considerable investigation. The tumor suppressor gene, p53, has been implicated, as has the epidermal growth factor receptor gene. Additional currently unidentified genes lie on chromosomes 10 and 19. Interestingly, work on pediatric astrocytomas suggests that the genes involved are different. p53 is rarely mutated in pediatric tumors, the epidermal growth factor receptor gene is rarely amplified or mutated, and chromosome 10 deletions are rare. The only pediatric tumor that seems to mimic the findings in adult tumors is brainstem glioma, perhaps explaining the uniformly grim prognosis in this type of tumor. In the pilocytic astrocytoma of childhood, mutations in the neurofibromatosis type I gene have been implicated in tumor development. In this review, the oncogenesis of pediatric gliomas is discussed and compared and contrasted to what is known about tumors.     

Gravity or suction drainage in thyroid surgery? Control of efficacy with ultrasound determination of residual hematoma

In a prospective randomized trial, the common high-vacuum drainage system according to Redon was compared with the nonsuction system according to Robinson in 80 patients undergoing elective thyroid surgery between January 1995 and August 1995. Forty patients were provided with nonsuction, passive drains, and another 40 patients were allocated to a control group with the high-vacuum system. Twenty-four h postoperatively, the wound area was analyzed by sonography after drainage removal. The dimension of the remaining hematoma was determined by scanning the operation field in six to seven layers (thickness per layer T = 1 cm). The area (A) of the hematoma was measured per layer, and thus the volume was determined by the formula: V = T x (A1 + A2.. + A(n)). Simultaneously, the quantity of discharge was determined. Patients receiving nonsuction drainage had significantly lower median drainage volume (34 ml; range: 0-175 ml vs-115 ml; range: 40-346 ml; P < 0.01) and a remaining hematoma, measured sonographically, of similar volume to that of the patients receiving high-vacuum treatment (4.4 ml; range: 0-21.7 ml vs 5.3 ml; range: 0.6-24.9 ml; not significant). No complications were observed. An advantage to using the nonsuction device is seen with respect to similar resting wound hematoma, lower fluid evacuation, and painless drain removal. This study supports prophylacity routine nonsuction wound drainage after elective thyroid surgery.     

Case report of pneumatic staple gun injury to the aorta

We describe an 8-year-old boy who had asymptomatic hypercalcemia 4 years after radiotherapy involving the left orbit and lungs. A right parathyroid adenoma was diagnosed, and normocalcemia was achieved after its removal. Routine monitoring of serum calcium and phosphate levels is recommended for children after head and neck irradiation.     

Medulloepithelioma of the optic nerve head

Medulloepithelioma of the optic nerve is a rare developmental tumor. We describe a 2-year-old boy with profound loss of vision associated with a visible tumor of the optic nerve head in his left eye. A clinically diagnosed retinoblastoma necessitated left eye enucleation. The histopathological diagnosis was malignant medulloepithelioma that was incompletely resected. Further tumor resection was required, and the patient received adjunctive chemotherapy and radiotherapy. Four years after treatment, the patient has neither clinical nor radiological evidence of tumor.     

Immunohistochemical analysis of metallothionein in astrocytic tumors in relation to tumor grade, proliferative potential, and survival

BACKGROUND: Metallothionein (MT) is the name for a family of predominantly intracellular protein thiol compounds involved in anticancer drug resistance. For certain tumors, MT is related to grade of tumor malignancy and prognosis. The authors evaluated the expression of MT in 114 astrocytic tumors in relation to the proliferative potential of tumors and the survival of patients. METHODS: Paraffin embedded tissue sections were stained with monoclonal anti-metallothionein and MIB-1 Ki-67 antibodies by avidin-biotin complex immunohistochemistry. RESULTS: MT expression was observed in 2 of 6 pilocytic astrocytomas, in 10 of 24 Grade 2 astrocytomas, in 16 of 25 anaplastic astrocytomas, and in 47 of 59 glioblastomas. In addition to the tumor cells, microvascular endothelial proliferation and smooth muscle of tumor vessel walls were frequently MT positive. The glioblastomas had a significantly higher percentage of MT positive cells compared with low grade (P < 0.0001) and anaplastic (P < 0.04) astrocytomas. MT expression in astrocytomas had no correlation with tumor recurrence. The mean Ki-67 labeling index (LI) was significantly higher in the high grade (3-4) compared with the low grade (1-2) astrocytomas. MT positive astrocytic tumors had statistically significantly higher mean Ki-67 LI compared with MT negative tumors, irrespective of histologic grade. Although the levels of MT and Ki-67 LI varied in individual tumors, the mean Ki-67 LI increased in parallel to the increasing MT staining grade, and this difference attained statistical significance only for glioblastoma. MT positive anaplastic astrocytoma and glioblastoma patients did not survive as long as the MT negative patients, although this difference attained statistical significance only for anaplastic astrocytoma. CONCLUSIONS: The current study suggests that MT might play a significant role in the growth of astrocytic tumors, with an acquired enhanced ability to produce MT as the malignant potential of a tumor increases.     

Chemotherapy for spinal cord astrocytoma: can natural history be modified?

Standard treatment of spinal cord astrocytomas is based upon surgery, followed by radiotherapy when resection is incomplete or when histology is of high grade. Owing to the major consequences of radiotherapy on the spine in childhood, alternative therapies must be explored. The potential role of chemotherapy in the management of spinal cord astrocytoma remains to be defined. Two patients are described. The first was a 19-month-old child with an anaplastic astrocytoma of the cervical spinal cord that progressed rapidly after initial partial resection. Chemotherapy was begun according to the UKCCSG Baby Brain Protocol, with marked clinical improvement. Reassessment by MRI at 4 months showed improvement, and at the end of treatment no evaluable disease remained. The second was a 4-year-old child with a recurrent low-grade astrocytoma. Chemotherapy according to the SIOP Protocol for Low Grade Gliomas was administered for 3 months, after which marked tumour regression was seen, with neurological recovery. These patients demonstrate the potential value and low morbidity of chemotherapy in spinal cord astrocytoma. The management of this rare tumour is discussed.     

Huge ossified crust-like subdural hematoma covering the hemisphere and causing acute signs of increased intracranial pressure

We report the successful removal of an ossified crust-like chronic subdural hematoma (SDH) covering the hemisphere in a 16-year-old boy. In this article, the importance of the surgical approach is stressed, and the rarity of this condition in the neurosurgical literature is also outlined.     

Magnetic resonance imaging changes after stereotactic radiation therapy for childhood low grade astrocytoma

BACKGROUND: Stereotactic radiotherapy (SRT) is fractionated radiotherapy delivered under stereotactic guidance to produce highly focal and precise therapy. We studied the incidence of imaging changes that can mimic tumor progression after completion of SRT for childhood low grade astrocytoma. METHODS: Between June 1992 and September 1994, we prospectively treated 28 children with low grade astrocytomas with SRT. The patients ranged in age from 2 to 22 years (median: 10 yrs) and none had received prior radiation therapy or radiosurgery. Routine fractionation was employed (180-200 centigray[cGy]) to a total dose of 5220-6000 cGy over 5 to 6 weeks. All of the patients underwent initial and follow-up magnetic resonance imaging (MRI) according to protocol. RESULTS: Median clinical follow-up for the 28 patients was 24 months (range, 5-32 mos) with a median radiographic follow-up of 15 months (range, 3-26 mos). Fifteen patients had reduction in tumor size, one patient had stable disease. Twelve patients (43%) developed increased size of the lesion, increased signal intensity or enhancement, cysts or cavitations, and an increase in edema or mass effect on follow-up MRI. Most of these changes occurred between 9 and 12 months after the start of SRT and resolved or decreased by 15 to 21 months. All but one patient had normal or stable neurologic examinations. CONCLUSIONS: Treatment-related MRI changes are common after conventionally fractionated schedules using stereotactic radiation techniques for patients with low grade astrocytomas. These changes can be distinguished from tumor progression by their transient nature as well as the general absence of clinical symptoms.